Adult Cerebellopontine Angle Medulloblastoma: A Systematic Review of Clinical Features, Management Approaches, and Patient Outcomes

Abstract

Objective: The aim of this study was to systematically review the existing individual patient data in the literature on adult cerebellopontine angle (CPA) medulloblastoma (MB) and characterize the patient presentation, management strategies used, and oncological outcomes of this rare entity to guide future clinical practice. Methods: Following PRISMA guidelines, a systematic review was conducted by searching PubMed, EMBASE, Web of Science, and Cochrane databases from inception to 19 June 2024. Studies regarding adult patients with histologically confirmed MB radiographically confirmed to be located in the CPA were included. Clinical data were synthesized, and predictors of outcomes were evaluated. Results: Twenty-seven studies with 42 adult CPAMB patients were included. The median age was 32 years (range: 19–56). Headaches (81%), cranial neuropathy (90%), cerebellar dysfunction (79%), and nausea/vomiting (50%) were typical presenting features. The predominant histological subtype was the classic variant. Maximal safe surgical resection was performed, most commonly using a retrosigmoid approach, and 60% of cases received a gross total resection. Most patients received adjuvant treatment (93%), typically chemoradiotherapy. The recurrence rate was 11% after a median of 18 months of follow-up. Relatively high survival rates of 96%, 85%, and 85% were observed at 1, 3, and 5 years, respectively. Patients who received adjuvant therapy had significantly better recurrence and greater overall survival outcomes. Conclusions: These results support the consideration of MB in young adult patients presenting with CPA tumors with radiographical features suggestive of hypercellularity and the utilization of a management strategy of maximal safe resection plus post-operative craniospinal irradiation along with chemotherapy to optimally treat these rare patients.